Synovial sarcoma: current perspectives

Bernadette Brennan Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK Abstract: Synovial sarcoma (SS) is a distinct soft tissue sarcoma, occurring across all ages, from young children to the elderly, but the incidence of SS peaks in young adults. Recently, its biology, specifically the biomarker genomic index, may prove to be the most important prognostic marker, explaining findings such as the positive effect of younger age on outcome. While believed to be a chemosensitive soft tissue sarcoma, surgery remains the most important modality of treatment for many people, especially in localized disease, plus or minus radiotherapy to improve local control. Moving forward, the demonstration that SS has multiple therapeutic targets such as vascular endothelial growth factor, and new emerging targets, allows us to start to consider different systemic therapeutic interventions other than just chemotherapy. This is particularly important for advanced/metastatic SS which, with conventional chemotherapy only, continues to have a very poor outcome. The way forward, therefore, is an all-age trial combining perhaps anti-angiogenesis agents with chemotherapy, focusing on those SS cases whose outcome, as determined by biology, site, or metastatic status, is poor with just conventional sarcoma chemotherapy. This also allows prospective evaluation of the role of genomic index and other biomarkers. Keywords: children/adolescents, genomic index, targeted therapy, MAMS design